Early-onset dementia neurologic features

Neurologic features	Range of diseases
Visual or ocular involvement Optic atrophy Retinal degeneration Cataracts Argyll Robertson pupil Kayser-Fleischer rings	Neurometabolic (MLD, Krabbe, PMD, CTX, HDLAS, mitochondrial disease) Neurometabolic (Krabbe, Cockayne syndrome, mitochondrial disease) Neurometabolic (CTX, mitochondrial disease) Neurosyphillis Wilson disease
Psychiatric or behavioral symptoms	FTD, DLB, neurometabolic (MLD, ALD, VWM, APBD, HDLAS)
Myoclonus or early seizures	Prion disease, AD, DLB, DRPLA, neurometabolic (mitochondrial disease, Gaucher disease, Tay-Sachs, ANCL, Lafora body disease, sialidosis), SSPE
Gaze palsy/ophthalmoplegia	PSP, SCA2, neurometabolic (Gaucher disease, Neimann-Pick type C, mitochondrial disease), Whipple disease, alcohol
Deafness	Superficial siderosis, mitochondrial disease
Dysautonomia	DLB, MSA, prion disease, anti-NMDAR encephalitis, AADL
Pyramidal signs	MS, FTD with MND, AD (PSEN1), SCA, neurometabolic (ALD, VWM, Krabbe, APBD, CTX, HDLAS)
Tremor	PDD, PSP, MSA, HIV, Wilson disease
Dystonia/chorea	HD, ANCL, CBD, Wilson disease, neuroacanthocytosis, Lesch-Nyhan, DRPLA, anti-NMDAR encephalitis, variant CJD
Peripheral neuropathy	HIV, alcohol, neurometabolic (CTX, ALD, MLD, Krabbe, APBD, mitochondrial disease, Fabry disease), SCA2
Abnormal gait and station	NPH, PDD, PSP, vascular dementia, neurosyphillis, Tay-Sachs
Ataxia	SCA, paraneoplastic encephalopathy, prion disease, DPRLA, fragile X, MSA, MS, neurometabolic (CTX, Alexander disease, APBD, VWM, mitochondrial disease, ANCL)
Migraine and stroke	CADASIL
Sleep disturbance	Neurodegenerative dementias, prion disease

MLD: metachromatic leukodystrophy; PMD: Pelizaeus-Merzbacher disease; CTX: cerebrotendinous xanthomatosis; HDLAS: hereditary diffuse leukoencephalopathy with axonal spheroids; FTD: frontotemporal dementia; DLB: dementia with Lewy bodies; ALD: X-linked adrenoleukodystrophy; VWM: vanishing white matter disease; APBD: adult polyglucosan body disease; AD: Alzheimer dementia; DRPLA: dentatorubral pallidoluysian atrophy; ANCL: adult-onset neuronal ceroid lipofuscinosis; SSPE: subacute sclerosing panencephalitis; PSP: progressive supranuclear palsy; SCA: spinocerebellar atrophy; MSA: multiple system atrophy; NMDAR: N-methyl-D-aspartate receptor; AADL: adult-onset autosomal dominant leukodystrophy; MS: multiple sclerosis; MND: motor neuron disease; PSEN1: presenilin 1; PDD: Parkinson disease dementia; HIV: human immunodeficiency virus; HD: Huntington disease; CBD: corticobasal degeneration; CJD: Creutzfeldt-Jakob disease; NPH: normal pressure hydrocephalus; CADASIL: cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy.

Adapted from:

Rossor MN, Fox NC, Mummery CJ, et al. The diagnosis of young-onset dementia. Lancet Neurol 2010; 9:793.
Ahmed RM, Murphy E, Davagnanam I, et al. A practical approach to diagnosing adult onset leukodystrophies. J Neurol Neurosurg Psychiatry 2014; 85:770.

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Early-onset dementia neurologic features